Dr. Bhosale Systemic

7/31/2019 Dr. Bhosale Systemic

1/87


2/87

Common systemic diseases

affecting the eye

Infectious

Toxoplasmosis

Toxocariasis

TBSyphilis

Leprosy

HIVCMV

Non-infectiousEndocrine diabetes,thyroid

Connective tissue

diseaseRA/SLE/Wegeners/PAN/Systemic sclerosis

Vasculities (GCA)

Sarcoidosis

Behcets DiseaseVogt Koyanagi Haradasyndrome

Phakomatoses


3/87

DIABETIC RETINOPATHY

1. Adverse risk factors

2. Pathogenesis

5. Clinically significant macular oedema

6. Preproliferative diabetic retinopathy

3. Background diabetic retinopathy

4. Diabetic maculopathies Focal Diffuse Ischaemic

7. Proliferative diabetic retinopathy


4/87

Location of lesions in backgrounddiabetic retinopathy


5/87

Signs of background diabetic retinopathy

Microaneurysmsusuallytemporal to foveaIntraretinal dot andblot haemorrhages

Hard exudatesfrequently

arranged in clumps orrings

Retinal oedemaseen asthickening on biomicroscopy


6/87

Preproliferative diabetic retinopathy

Treatment - not required but watch for proliferative disease

Cotton-wool spots

Venous irregularities

Dark blot haemorrhages

Intraretinal microvascularabnormalities (IRMA)

Signs


7/87

Proliferative diabetic retinopathy

Flat or elevated

Severity determined by comparing with area of disc

Neovascularization

Neovascularization of disc = NVD

Affects 5-10% of diabetics IDD at increased risk (60% after 30 years)

Neovascularization elsewhere = NVE


8/87

Spot size (200-500 m) dependson contact lens magnification

Gentle intensity burn (0.10-0.05 sec)

Follow-up 4 to 8 weeks

Area covered by complete PRP Initial treatment is 2000-3000 burns

Laser panretinal photocoagulation


9/87

Retinal Vein Occlusion

Second most common cause of vascular-related visual loss.

Risk factors: hypertension, age, blood dyscrasias (OCP,HRT) and

vasculitis (Behcets,sarcoidosis,AIDS,SLE)


10/87

Retinal Artery Occlusion

Risk factors: Carotid artery atherosclerosis (CRAO), carotid emboli

(BRAO), vasculitis (GCA,SLE,PAN), coagulopathy.


11/87

1. Soft tissue involvement Periorbital and lid swelling

Conjunctival hyperaemia

Chemosis

Superior limbic keratoconjunctivitis

2. Eyelid retraction

3. Proptosis

4. Optic neuropathy

5. Restrictive myopathy

THYROID EYE DISEASE


12/87

Soft tissue involvementPeriorbital and lid swelling

Chemosis

Conjunctival hyperaemia

Superior limbickeratoconjunctivitis


13/87

Signs of eyelid retractionOccurs in about 50%

Bilateral lid retraction

No associated proptosis

Bilateral lid retraction

Bilateral proptosis

Lid lag in downgaze Unilateral lid retraction

Unilateral proptosis

i


14/87

Proptosis

Treatment options Systemic steroids

Radiotherapy Surgical decompression

Occurs in about 50% Uninfluenced by treatment of hyperthyroidism

Axial and permanent in about 70% May be associated with choroidal folds


15/87

Optic neuropathy Occurs in about 5% Early defective colour vision

Usually normal disc appearance

Caused by optic nervecompression atorbital apex by enlarged recti

Often occurs in absence of significantproptosis

R i i h


16/87

Occurs in about 40% Due to fibrotic contracture

Restrictive myopathy

Elevation defect - most common Abduction defect - less common

Depression defect - uncommon Adduction defect - rare


17/87

SARCOIDOSIS

Idiopathic multisystem disorder

Characterised by non-caseating

granulomata

More common in women 20-50 yrs

More common in blacks and Asians

? Related to mycobacteria


18/87

SARCOIDOSIS

Systemic InvolvementLung lesions 95%

Thoracic lymph nodes

50%

Skin lesions 30%

Eyes 30%


19/87

SARCOIDOSIS

Ocular InvolvementAnterior segmentlesions (30%)

Conjunctival granuloma

Lacrimal gland

involvement/dry eye Acute or chronic uveitis

KPs described asmutton fat because they

are large and greasy


20/87

SARCOIDOSIS

Ocular InvolvementPosterior segmentlesions (20%) Patchy venous sheathing

Cellular infiltrate aroundvessels

Chorioretinalgranulonmas

Vasculitis includingocclusive causing:-

Neovascularisation

Infiltrate in vitreous(vitritis) including cellclumps (snowballs)


21/87

SARCOIDOSIS

Ocular InvolvementSheathing of the

retinal veins

Fluoresceinangiography showing

leakage and staining

at sites of sheathing


22/87

SARCOIDOSIS

Granuloma in Fundus

Retinal and pre-

retinal

Choroidal


23/87

SARCOIDOSIS

Granuloma in FundusOptic nerve head

granuloma

Normal optic nervehead


24/87

SARCOIDOSIS

Systemic SignsLupus pernio affecting

the nose a chronic

progressive

cutaneous sarcoidthat most commonly

affects face and ears


25/87

SARCOIDOSIS

Systemic signsFacial palsy

Salivary gland

enlargement


26/87

SARCOIDOSIS

Systemic signsHilar adenopathy on

chest x-ray

Lung infiltrate

Erythema nodosum

Arthritis


27/87

SARCOIDOSIS

Investigations (1)CXR to detect

pulmonary signs

Bilateral hilar lymph-

adenopathy

Pulmonary mottling


28/87

SARCOIDOSIS

Investigations (2)

Serum angiotensin-converting enzyme

(ACE) elevated in active sarcoidosis

Mantoux test caution in patients who

have had BCG vaccination. Test may be

negative

Lung function tests


29/87

SARCOIDOSIS

Investigations (3)Gallium scan showing

increased uptake in

the lacrimal and

parotid glands andpulmonary regions in

a patient with active

sarcoidosis


30/87

SARCOIDOSIS

Treatment

Systemic steroids may be necessary in

patients with posterior segment disease

where vision is threatened, especially if

optic nerve is involved


31/87

PHACOMATOSES

1. Neurofibromatosis

2. Tuberous sclerosis (Bourneville disease)

3. von-Hippel-Lindau syndrome

4. Sturge-Weber syndrome

Type I (NF-1) - von Recklinghausen disease

Type II (NF-2) - bilateral acoustic neuromas

Neurofibromatosis type 1 (NF 1)


32/87

Neurofibromatosis type-1 - (NF-1)

Appear during first year of life

Caf-au-lait spots

Most common phacomatosis

Increase in size and number throughoutchildhood

Affects 1:4000 individuals

Presents in childhood Gene localized to chromosome 17q11

Fibroma molluscum in NF 1


33/87

Fibroma molluscum in NF-1

Appear at puberty

Pedunculated, flabby nodulesconsisting of

neurofibromas or schwannomas

Increase in numberthroughout life

Frequently widely distributed


34/87

Plexiform neurofibroma in NF-1

May be associated withovergrowth of overlying skin

Appear during childhood Large and ill-defined

Skeletal defects in NF 1


35/87

Skeletal defects in NF-1

Mild head enlargement - uncommon Other - scoliosis, short stature, thinning of

long bones

Facial hemiatrophy

Orbital lesions in NF 1


36/87

Orbital lesions in NF-1

Spheno-orbital encephaloceleOptic nerve glioma in about 15%

Sagittal MRI scan of optic nerve

glioma invading hypothalamus

Glioma may be unilateral or

bilateral

Axial CT scan of congenital absence ofleft greater wing of sphenoid bone

Causes pulsating proptosis without bruit


37/87

Eyelid neurofibromas in NF-1

Nodular Plexiform

May cause mechanical ptosis May be associated with glaucoma

Intraocular lesions in NF-1


38/87

Intraocular lesions in NF-1Lisch nodules

Very common - eventually presentin 95% of cases

Congenital ectropion uveae

Uncommon - may be associatedwith glaucoma

Retinal astrocytomas

Rare - identical to those seen in

tuberous sclerosis

Choroidal naevi

Common - may be multifocal

and bilateral


39/87

Ocular features of NF-2

Common - combined hamartomas of RPand retina

Very common -presenile cataract

Tuberous sclerosis (Bourneville disease)


40/87

Tuberous sclerosis (Bourneville disease)

Diffuse thickening overlumbar region

Present in 40%

Shagreen patches

Autosomal dominant Triad - mental handicap, epilepsy, adenoma sebaceum

Adenoma sebaceum

Around nose andcheeks

Appear after age 1

and slowly enlarge

Ash leaf spots

Hypopigmented skin patches

In infants best detected usingultraviolet light (Woods lamp)

Systemic hamartomas in tuberous sclerosis


41/87

Systemic hamartomas in tuberous sclerosisAstrocytic cerebral hamartomas

Slow-growing periventricular tumours

May cause hydrocephalus, epilepsy andmental retardation

Usually asymptomatic andinnocuous

Kidneys (angiomyolipoma), heart

(rhabdomyoma)

Visceral and subungual hamartomas

R ti l t t i t b l iti


42/87

Retinal astrocytomas in tuberous scleritis

Dense white tumour Mulberry-like tumour

Early

Innocuous tumour present in 50% of patients May be multiple and bilateral

Semitranslucent nodule White plaque

Advanced

Systemic features of v-H-L syndrome


43/87

Systemic features of v H L syndromeAutosomal dominant

Tumours - renalcarcinoma and

phaeochromocytoma

Cysts - kidneys, liver,pancreas, epididymis,ovary and lungs

Polycythaemia

CNS Haemangioblastoma

MRI of spinal cord tumour

Angiogram of cerebellartumour

Visceral tumours

Retinal capillary haemangioma


44/87

Retinal capillary haemangiomain v-H-L syndrome

Round orange-red mass

Early

Vision-threatening tumour present in 50% of patients May be multiple and bilateral

Tiny lesion betweenarteriole and venuole

Small red nodule

Associated dilatation and

tortuosity of feeder vessels

Advanced

Systemic features of Sturge Weber syndrome


45/87

Systemic features of Sturge-Weber syndrome

Congenital, does not blanchewith pressure

Associated with ipsilateralglaucoma in 30% of cases

Naevus flammeus

CT scan showing leftparietal haemangioma

Complications - mental handicap,epilepsy and hemiparesis

Meningeal haemangioma

Ocular features of Sturge-Weber syndrome


46/87

Ocular features of Sturge Weber syndrome

Normal eye

Buphthalmos in 60% May be associated with

episcleral haemangioma

Affected eye

Diffuse choroidal haemangioma

Glaucoma

Peripheral corneal involvement in


47/87

Peripheral corneal involvement inrheumatoid arthritis

Chronic and asymptomatic

Circumferential thinning with intactepithelium (contact lens cornea)

Acute and painful

Circumferential ulceration andinfiltration

Treatment - systemic steroids and/or cytotoxic drugs

Without inflammation With inflammation

Peripheral corneal involvement in


48/87

Peripheral corneal involvement inegener granulomatosis and polyarteritis nodo

Circumferential and centralulceration similar to Mooren ulcer

Unlike Mooren ulcer sclera may alsobecome involved

Treatment - systemic steroids and cyclophosphamide


49/87

GIANT CELL ARTERITIS


50/87


PresentationHeadache

Scalp tenderness

Thickened temporal

arteriesJaw claudication

Acute visual loss

Weight loss, anorexia,

fever, night sweats,malaise & depression



51/87


Ocular ComplicationsTransient monocularvisual loss (amaurosisfugax)

Visual loss due to Central retinal artery

occlusion (CRAO) or

Anterior ischaemicoptic neuropathy

(AION)Visual field defects



52/87


ManagementESR if suspected

Start high dose steroids immediately to

prevent stroke or second eye involvement

Temporal artery biopsy within a week of

starting steroids



53/87


Temporal Artery BiopsyArteries have skiplesions

ultrasound/Doppler mayhelp identify involved

areasIf positive, confirmsdiagnosis helpful inmanagement of futuredisease

If negative, doesntexclude diagnosis, butneed to think about analternative diagnosis



54/87


HistopathologyGranulomatous cell

infiltration

Giant cells

Disruption of internalelastic lamina

Proliferation of intima

Occlusion of lumen



55/87


Treatment

Intravenous and oral steroids prolonged

course of steroids often necessary


56/87

Ocular manifestations of

HIV infection

I t d ti


57/87

Introduction

AIDS is an infectious disease caused by the gradual

decrease in CD4+ T lymphocytes causing

subsequent opportunistic infections and neoplasia. It

is a blood borne and sexually transmitted infection

caused by the HIV (Human Immunodeficiency Virus)Approximately 36 million persons around the world

are infected. Up to 70% of patients infected with HIV

will develop some form of ocular involvement, ie:

direct infection by HIV,opportunistic infections andneoplasia.

HIV infection progresses though different phases


58/87

Ophthalmic Manifestations of HIV Infection

AROUND THE EYE

Molluscum Contagiosum

Herpes Zoster

Ophthalmicus

Kaposis Sarcoma Conjunctival Squamous

Cell Carcinoma

Trichomegaly

FRONT OF THE EYE

Dry Eye

Anterior Uveitis

BACK OF THE EYE

Retinal Microvasculopathy

CMV Retinitis

Acute Retinal Necrosis

Progressive Outer RetinalNecrosis

Toxoplasmosis

Retinochoroiditis

Syphilis Retinitis

Candida albicansendophthalmitis

NEURO-OPHTHALMIC

M ll C t i


59/87

Molluscum Contagiosum

Molluscum contagiosum is a

viral infection of the skin.

Affects up to 20% of

symptomatic HIV infected

patients.

Clinically appears like painless,

small, umbilicated nodules,

which produce a waxy

discharge when pressured.

Treatment consists on excision

of the lesion, curettage or

cryotherapy

Herpes Zoster Ophthalmicus


60/87

Herpes Zoster Ophthalmicus

Due to the reactivation of a latent infection by Varicella

Zoster Virus in the dorsal root of trigeminal nerve

ganglion.

It manifests with a maculo-papulo-vesicular rash whichoften is preceded by pain. Usually involves the upper lid

and does not cross the midline

Treatment consists on oral Aciclovir 800mg 5 times

/day. In immunocompromised patients Aciclovir is givenintravenously for two weeks. Ocular manifestations

such as anterior uveitis, are treated with topical steroids

and mydriatics.


61/87

Kaposis Sarcoma


62/87

Kaposi s Sarcoma

Kaposis sarcoma is a vascular neoplasm which is almost

exclusively seen in patients with AIDS.

KS is the commonest anterior segment lesion seen in AIDS;

appears as a violaceous non-tender nodule on the eyelid or

conjunctiva.

Typically KS involves only the skin but when there is a

reduced CD4 count it can progress rapidly to other sites

such as the gastrointestinal tract and CNS

Treatment of ocular adnexal KS may be necessary for

cosmesis and to relieve functional difficulties. The mainstay

of treatment is radiotherapy. Other options include

cryotherapy or chemotherapy.


63/87


64/87

Conjunctival Squamous Cell Carcinoma

Squamous cell carcinoma (SCC) is the third mostcommon neoplasm associated to HIV infection. This may

be due to an interaction between HIV, sunlight and

Human Papilloma Virus infection.

SCC appears as a pink, gelatinous growth, usually in theinterpalpebral area. Often an engorgedblood vesselfeeding the tumour is seen. It may extend onto the

cornea, but deep invasion and metastasis are rare.

The treatment of choice is local excision and cryotherapybut the presence of orbital invasion is an indication of

exenteration


65/87


66/87

Trichomegaly

Trichomegaly orhypertrichosis is an

exaggerated growth of

the eye lashes found in

the later stages of thedisease

The cause is not known

When symptomatic or for

cosmetic reasons theeyelashes can be

trimmed or plucked


67/87

Dry EyeSicca syndrome is

frequent amongpatients with HIV

infection

Patients complain of

burning uncomfortablered eyes.

There are several

causes of dry eye in

HIV infection from

blepharitis to

destruction of the

lacrimal glands.

Treatment is with tear

supplements

Anterior Uveitis


68/87

Anterior Uveitis

HIV related anterioruveitis can

be: Direct manifestation of the

human immunodeficiency

virus infection

autoimmnune in origin drug induced ie: rifabutin,

secondary to direct toxic

effect upon the non-

pigmented epithelium of the

ciliary body

Any of the different infections

associated with AIDS, ie:

Herpes Zoster Virus, Herpes

Simplex Virus,

Rifabutin induced anterior uveitis


69/87

Rifabutin induced anterior uveitis


70/87

Retinal microvasculitis

Retinal microvasculopathy occurs in more than half of thepatients with HIV

It is seen as transient cotton wool spots (CWS), intra-retinal

haemorrhages and microaneurysm, which occurs in 50-70% of

patients. It is usually asymptomatic.It has an unclear pathogenesis, but it is thought to be HIV

infection of retinal vascular cells.

In an otherwise healthy individual the presence of CWS, should

be differentiated from other forms of retinopathy, such asdiabetic or hypertensive retinopathy. Serological test for HIV will

confirm the diagnosis

Treatment is based in delaying the progression of the disease

associated with HIV


71/87

Cotton Wool Spots

CMV Retinitis


72/87

CMV Retinitis

Introduction

CMV Retinitis is the commonest intraocular ocular opportunistic infectionseen in patients with AIDS

Antibodies are found in almost 95% of adults, causing a trivial illness in

immunocompetent adults, however severe immunosuppression causes

viral reactivation and tissue invasive disease

Pathogenesis

Reactivation from extraocular sites leads to seeding in other sites such

as the retina

Epidemiology

The number of newly diagnosed cases of CMVR has decreased sincethe introduction of the HAART

Highly Active Antiretroviral Therapy

CMV Retinitis


73/87

CMV Retinitis

Clinical manifestations

Patients may complain of minor visual symptoms such as floaters,flashing lights or mild blurred vision, or be totally asymptomatic.

It presents with a wide range ofclinical appearances. From cotton wool

spots which may look like HIV Retinopathy to confluent areas of full

thickness retinal necrosis and vasculitis. CMVR can progress in a

brushfire pattern from the active edge of an active lesion. The retinalvessels in an affected area show attenuation, becoming ghost vessels

eventually.

Treatment

The treatment of CMVR in patients with AIDS requires the use of specific

antiviral agents, ganciclovir, foscarnet or cidovir in conjunction withHAART.

These treatments can be administered orally, intravenously or

intravitreally. Systemic treatment has the advantage of treating infection

elsewhere in the body as well as the other eye but has the

disadvantages of systemic side effects.

CMV Retinitis


74/87

CMV Retinitis



75/87


ARN is a confluent peripheral whitening of the retina withmarked vitritis and blood vessel closure. Optic neuritis

and retinal detachment are frequent complications.

ARN is usually due to Varicella-Zoster infection, but it can

also be caused by Herpes Simplex virus or

Cytomegalovirus.

Initially described in the immunocompetent, it has also

been described in the immunosuppressed.

The diagnosis is mainly clinical and is confirmed by PCR

assays on vitreous samples.

Patients are treated with high doses of intravenous

aciclovir or famciclovir, combined with laser treatment to

prevent retinal detachment.



76/87


rogress ve u er e naNecrosis


77/87

Necrosis

(Varicella-Zoster Retinitis)

PORN is a devastating viral retinitis caused by Varicella-Zostervirus, without vitritis or retinal vasculitis.

The retinitis can be located anywhere but it is common for the

lesions to coalesce and spread posteriorly in a rapid fashion.

The main symptom is rapid loss of vision.The retina showstypically a white lesion with no haemorrhages or exudates.

Treatment is often unsatisfactory and usually requires

combination of Ganciclovir and Aciclovir. The prognosis is very

poor and retinal detachment is common. Resolution may leavea white plaque with the appearance of cracked mud.


78/87

Toxoplasma Retinochoroiditis


79/87


Toxoplasmosis retinochoroiditis is an uncommoninfection of the eye in AIDS. Ocular toxoplasmosis in HIV

positive patients is different in appearance from

immunocompetent patients. Unlike in immunocompetent

patients, HIV infected patients often have bilateral andmultifocal disease associated with anterior uveitis and

vitritis but unlike immunocompetent patients, in HIV

infected patients often have with no pigmented scars

adjacent to the areas of retinal necrosis. Toxoplasmosis

in immunocompromised patients is not self-limiting as it

is in imunocompetent patients.



80/87


When testing patients for antibodies to toxoplasmosisboth IgG and IgM levels may be raised, but in

immunocompromised patients these tests may be

negative.

Treatment in immunocompromised patients consists inthe association of sulphadiazine or clindamycin,

pyrimethamine and folinic acid (triple therapy).

Long term maintenance treatment may be needed in

order to prevent relapses.Often associated with toxoplasma lesions in the Central

Nervous System.


81/87

MRI T1 showing an uniformly

enhancing lesion in the

midbrain

One week later, the lesion

showing ring enhancement


82/87

Immunocompetent Immunocompromised

Syphilis Retinitis


83/87

Syphilis Retinitis

There is a strong association between syphilis andHIV infection.

It can manifest as a retinitis with dense vitritis,

retinal vasculitis, serous retinal detachment or

neuroretinitis, as well as other types of ocularinvolvement such as, conjunctivitis, anterior uveitis,

cranial nerve palsies and optic neuritis.

Treatment consists in high dose of intravenous

Penicillin for 2 weeks.


84/87

Candida albicans

d hth l iti


85/87

endophthalmitis

Infection with candida albicans is rare. Candida albicansis the commonest cause of fungal endophthalmitis

Affected patients usually have a history of drug abuse

or indwelling central lines

In the initial stages, floaters are the main symptom. Asthe condition progresses, whitish puff-balls and

vitreous strands develop. Later, similar infiltrates appear

in the choroid and retina

The treatment depends on the severity of the ocularinvolvement and systemic disease. The original foci

should be removed. The drugs of choice are

Amphotericine B and Fluconazol

Candida albicans

d hth l iti


86/87

endophthalmitis

Glossary


87/87

Glossary

CD4: Director of the immune response. When activated it

releases cytokines which in turn will activate the immunesystem

Cotton Wool Spots: Light-coloured deposits in the retinasecondary to infarcts of the nerve fibre layer

HAART: Highly Active Antiretroviral TherapyImmunoblogulin: Protein in charge of fighting foreignsubstances in our body. IgG is the commonest type of

immunoglobulin and IgM is the earliest class

of immunoglobulin.PCR: Polymerase Chain Reaction is a technique used to make

numerous copies of an specific portion of DNA

VDRL: Venereal Disease Research Laboratory The test

Dr. Bhosale Systemic

Documents

Transcript of Dr. Bhosale Systemic